Peripheral Nerve Disorders 2

Author: Arthur K. Asbury
Publisher: Butterworth-Heinemann Medical
ISBN:
Size: 26.48 MB
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Peripheral Nerve Disorders 2 from the Author: Arthur K. Asbury. The flow of new information in the eleven years since the publication of the first volume (published in the BIMR series), has long necessitated a new look at peripheral nerve disorders. Thus this new volume emphasises the advances of the past decade and includes information on newly recognized neuropathies, hereditary neuropathies, peripheral nerve dysfunction and therapeutic advances. It has over 20 contributors from the UK, USA and continental Europe.

Peripheral Nerve Disorders

Author: GĂ©rard Said
Publisher: Elsevier
ISBN: 0444633553
Size: 13.60 MB
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Peripheral Nerve Disorders from the Author: GĂ©rard Said. Disorders of the peripheral nervous system (PNS) are the cause of prominent neurological symptoms including weakness, sensory loss, pain and autonomic dysfunction associated with deficits, morbidity and mortality. These disorders may be primary hereditary or cryptogenic neurologic disorders confined to the PNS or part of the pathology of both the central nervous system and the PNS. Most PNS disorders are secondary to other system disorders and may be responsive to treatment of the primary disease. Important advances have been obtained in several areas including molecular genetics, biochemistry, immunology, morphology and physiology that have enhanced our understanding of the causes and consequences of damage to peripheral nerve. Understanding of both these groups of PNS diseases has greatly expanded over recent years and has led to important advances of treatment both to protect and to repair damages of peripheral nerve. This volume provides an overview of the state-of-the-art of examination, diagnosis and treatment of these very diverse disorders and will be of interest to both the research and clinical neuroscience and neurology communities. Covers both hereditary and cryptogenic neurologic disorders Includes advances in the basic science of PNS from molecular genetics, biochemistry, immunology, morphology and physiology Detailed coverage of neuropathy in connective tissue disorders, infectious disorders, metabolic disorders and malignancy

Peripheral Nerve Disorders

Author: Simon K.W. Lloyd
Publisher: Elsevier Inc. Chapters
ISBN: 0128078855
Size: 71.48 MB
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Peripheral Nerve Disorders from the Author: Simon K.W. Lloyd. Neurofibromatosis type 2 (NF2) is an autosomal dominant inherited tumor predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. Affected individuals develop schwannomas typically involving both vestibular nerves leading to hearing loss and eventual deafness. Rehabilitation with brainstem implants and in some cases cochlear implants is improving this outcome. Schwannomas also occur on other cranial nerves, on spinal nerve roots and peripheral nerves, and intracutaneously as plaques. Cranial and spinal meningiomas and spinal ependymomas are other common tumors. Fifty to sixty percent of patients represent de novo mutations and as many as 33% of these are mosaic for the underlying disease causing mutation. Truncating mutations (nonsense, frameshift insertions/deletions) are the most frequent germline events and cause the most severe disease, whilst single and multiple exon deletions are common and are usually associated with milder NF2. Neurological deficits are a major feature of the condition and neurologists have a pivotal role in assigning symptoms to lesions and in managing neuropathies. NF2 represents a difficult management problem with most patients facing substantial morbidity and reduced life expectancy. Surgery remains the focus of current management although watchful waiting and occasionally radiation treatment have a role. We are seeing the advent of tailored drug therapies aimed at the genetic level and these are likely to provide huge improvements for this devastating, life-limiting condition.

Peripheral Nerve Disorders

Author: Jean-Michel Vallat
Publisher: John Wiley & Sons
ISBN: 1118618432
Size: 44.86 MB
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Peripheral Nerve Disorders from the Author: Jean-Michel Vallat. Peripheral Nerve Disorders: Pathology and Genetics is a definitive, clinically-oriented guide to the pathology of peripheral nerve disorders. These commonly seen neurological challenges have many causes and accurate diagnosis is often necessary via pathological analysis. New techniques exploiting molecular biological knowledge have opened up new vistas to understanding the pathogenesis of these disorders, and hence their effective management. This new title takes a disease-oriented approach to understanding the pathology of these conditions. It combines classical and contemporary techniques to enable practitioners in neurology and neuropathology to better understanding of the disease processes underlying patients’ presentations and to formulate appropriate management plans. Peripheral Nerve Disorders: Pathology and Genetics is a valuable resource for neurologists, neuropathologists, pathologists, neurobiologists and geneticists.

Peripheral Nerve Disorders

Author: José Berciano
Publisher: Elsevier Inc. Chapters
ISBN: 0128078820
Size: 33.62 MB
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Peripheral Nerve Disorders from the Author: JosĂ© Berciano. Hereditary ataxias (HA) encompass an increasing number of degenerative disorders characterized by progressive cerebellar ataxia usually accompanied by extracerebellar semeiology including peripheral nerve involvement. Classically, HA were classified according to their pathological hallmark comprising three main forms: (1) spinal form predominantly with degeneration of spinocerebellar tracts, posterior columns, and pyramidal tracts (Friedreich’s ataxia, FA); (2) olivopontocerebellar atrophy (OPCA); and (3) cortical cerebellar atrophy (CCA). In the 1980s Harding proposed a clinico-genetic classification based upon age of onset, modality of transmission, and clinical semeiology. The main categories in this classification were as follows: (1) early onset cerebellar ataxia (EOCA) with age of onset below 25 years and usually with autosomal recessive (AR) transmission (this group encompasses FA and syndromes different from FA); (2) autosomal dominant cerebellar ataxia (ADCA) with adult onset and with either cerebellar-plus syndrome or pure cerebellar semeiology; and (3) idiopathic late onset onset cerebellar ataxia (ILOCA). With the advent of molecular genetics, the nosology of HA has been in a state of constant flux. At present EOCA comprises at least 17 genotypes (designated with the acronym of ARCA derived from AR cerebellar ataxia), whereas under the umbrella of ADCA 30 genotypes have been reported. In this chapter we will review peripheral nerve involvement in classical pathological entities (OPCA and CCA), ARCA, ADCA, and ILOCA paying special attention to the most prevalent syndromes in each category. As a general rule, nerve involvement is relatively common in any form of ataxia except ILOCA, the most common pattern being either sensory or sensorimotor neuronopathy with a dying-back process. An exception to this rule is AR spastic ataxia of Charlevoix–Saguenay where nerve conduction studies show the characteristic pattern of intermediate neuropathy implying that sacsin mutation causes both axonal and Schwann cell dysfunction.

Peripheral Nerve Disorders

Author: Israel Steiner
Publisher: Elsevier Inc. Chapters
ISBN: 0128078626
Size: 54.43 MB
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Peripheral Nerve Disorders from the Author: Israel Steiner. Among the human herpes viruses, three are neurotropic and capable of producing severe neurological abnormalities: herpes simplex virus type 1 and 2 (HSV-1 and HSV-2) and varicella-zoster virus (VZV). Both the acute, primary infection and the reactivation from the site of latent infection, the dorsal sensory ganglia, are associated with severe human morbidity and mortality. The peripheral nervous system is one of the major loci affected by these viruses. The present review details the virology and molecular biology underlying the human infection. This is followed by detailed description of the symtomatology, clinical presentation, diagnosis, course, therapy, and prognosis of disorders of the peripheral nervous system caused by these viruses.

Peripheral Nerve Disorders

Author: Sinéad M. Murphy
Publisher: Elsevier Inc. Chapters
ISBN: 012807843X
Size: 70.19 MB
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Peripheral Nerve Disorders from the Author: Sinéad M. Murphy. The inherited neuropathies are a clinically and genetically heterogeneous group of disorders in which there have been rapid advances in the last two decades. Molecular genetic testing is now an integral part of the evaluation of patients with inherited neuropathies. In this chapter we describe the genes responsible for the primary inherited neuropathies. We briefly discuss the clinical phenotype of each of the known inherited neuropathy subgroups, describe algorithms for molecular genetic testing of affected patients and discuss genetic counseling. The basic principles of careful phenotyping, documenting an accurate family history, and testing the available genes in an appropriate manner should identify the vast majority of individuals with CMT1 and many of those with CMT2. In this chapter we also describe the current methods of genetic testing. As advances are made in molecular genetic technologies and improvements are made in bioinformatics, it is likely that the current time-consuming methods of DNA sequencing will give way to quicker and more efficient high-throughput methods, which are briefly discussed here.

Peripheral Nerve Disorders

Author: Jean-Michel Vallat
Publisher: John Wiley & Sons
ISBN: 1118618416
Size: 56.51 MB
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Peripheral Nerve Disorders from the Author: Jean-Michel Vallat. Peripheral Nerve Disorders: Pathology and Genetics is a definitive, clinically-oriented guide to the pathology of peripheral nerve disorders. These commonly seen neurological challenges have many causes and accurate diagnosis is often necessary via pathological analysis. New techniques exploiting molecular biological knowledge have opened up new vistas to understanding the pathogenesis of these disorders, and hence their effective management. This new title takes a disease-oriented approach to understanding the pathology of these conditions. It combines classical and contemporary techniques to enable practitioners in neurology and neuropathology to better understanding of the disease processes underlying patients’ presentations and to formulate appropriate management plans. Peripheral Nerve Disorders: Pathology and Genetics is a valuable resource for neurologists, neuropathologists, pathologists, neurobiologists and geneticists.

Peripheral Nerve Disorders

Author: GĂ©rard Said
Publisher: Elsevier Inc. Chapters
ISBN: 0128078448
Size: 25.24 MB
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Peripheral Nerve Disorders from the Author: GĂ©rard Said. Examination of a patient with peripheral neuropathy starts with careful questioning of the patient about the history of symptoms and signs and of a possible familial disorder. Several steps are required during examination of the patient with peripheral neuropathy: first the pattern of neuropathy and site of lesions should be determined: roots, nerve trunks, focal, multifocal, length-dependent generalized polyneuropathy, the type of nerve fibers predominantly affected, the association with trophic changes and autonomic dysfunction, the course of the disease ranging from acute inflammatory polyneuritis or fulminant multifocal neuropathy to an extremely slow progression as in Charcot−Marie−Tooth syndromes. At the end of this first contact with the patient, the neurologist must decide which investigations seem necessary and their timing including electrophysiological tests, imaging, CSF examination, blood tests, nerve and muscle biopsy, DNA testing, etc. In some cases, life-threatening manifestations, including weakness of respiratory muscles or swallowing difficulty, or autonomic dysfunction, require urgent therapeutic decisions.

Peripheral Nerve Disorders

Author: Andrea M. Vincent
Publisher: Elsevier Inc. Chapters
ISBN: 0128078650
Size: 62.63 MB
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Peripheral Nerve Disorders from the Author: Andrea M. Vincent. More than half of all patients with diabetes develop neuropathic disorders affecting the distal sensory and/or motor nerves, or autonomic or cranial nerve functions. Glycemic control can decrease the incidence of neuropathy but is not adequate alone to prevent or treat the disease. This chapter introduces diabetic neuropathy with a morphological description of the disease then describes our current understanding of metabolic and molecular mechanisms that contribute to neurovascular dysfunctions. Key mechanisms include glucose and lipid imbalances and insulin resistance that are interconnected via oxidative stress, inflammation, and altered gene expression. These complex interactions should be considered for the development of new treatment strategies against the onset or progression of neuropathy. Advances in understanding the combined metabolic stressors and the novel study of epigenetics suggest new therapeutic targets to combat this morbid and intractable disease affecting millions of patients with type 1 or type 2 diabetes.